TY - JOUR
T1 - Orbital involvement in sickle cell disease
T2 - A report of five cases and review literature
AU - Ganesh, Anuradha
AU - William, Ranjan R.
AU - Mitra, Sandip
AU - Yanamadala, Sujatha
AU - Hussein, Samir S.
AU - Al-Kindi, Salam
AU - Zakariah, Mathew
AU - Al-Lamki, Zakia
AU - Knox-Macaulay, Huxley
PY - 2001
Y1 - 2001
N2 - Purpose. To present five cases of orbital infarction in sickle cell disease and review relevant literature. Method. We reviewed the hospital records of 5 patients with sickle cell disease who developed a periorbital swelling during a vaso-occlusive crisis and were managed at our hospital between April 1992 and June 2000. Results. The 5 patients (4 with homozygous sickle cell disease and 1 with sickle cell-β-thalassaemia disease) were aged 6-15 years with a history of multiple admissions for vaso-occlusive crises. The periorbital swelling spread to the orbit in 4 cases and resulted in proptosis (2 cases), restriction of ocular motility and visual impairment. In all 4 cases, computed tomography and/or magnetic resonance imaging of the orbits showed a mass adjacent to the orbital wall. In 2 cases the mass was identified as a haematoma. Orbital wall infarction was demonstrated in 3 cases by bone/bone marrow scintigraphy. Epidural haematomas were detected by computed tomography in one case. All patients received intravenous fluids, analgesics, broad spectrum antibiotics and steroids, as well as simple or exchange transfusion, and responded well to medical management. Conclusions. Infarction of orbital bones during vaso-occlusive crises in sickle cell disease presents acutely with a rapidly progressive periorbital swelling. Haematomas frequently complicate the condition and, along with the inflammatory swelling, may lead to orbital compression syndrome. The condition is therefore sight-threatening, and necessitates prompt diagnosis and appropriate management for resolution without adverse sequelae. Imaging techniques are invaluable in the evaluation of patients. The majority of cases resolve with conservative treatment that includes steps to combat the vaso-occlusive crisis and use of systemic steroids under antibiotic cover.
AB - Purpose. To present five cases of orbital infarction in sickle cell disease and review relevant literature. Method. We reviewed the hospital records of 5 patients with sickle cell disease who developed a periorbital swelling during a vaso-occlusive crisis and were managed at our hospital between April 1992 and June 2000. Results. The 5 patients (4 with homozygous sickle cell disease and 1 with sickle cell-β-thalassaemia disease) were aged 6-15 years with a history of multiple admissions for vaso-occlusive crises. The periorbital swelling spread to the orbit in 4 cases and resulted in proptosis (2 cases), restriction of ocular motility and visual impairment. In all 4 cases, computed tomography and/or magnetic resonance imaging of the orbits showed a mass adjacent to the orbital wall. In 2 cases the mass was identified as a haematoma. Orbital wall infarction was demonstrated in 3 cases by bone/bone marrow scintigraphy. Epidural haematomas were detected by computed tomography in one case. All patients received intravenous fluids, analgesics, broad spectrum antibiotics and steroids, as well as simple or exchange transfusion, and responded well to medical management. Conclusions. Infarction of orbital bones during vaso-occlusive crises in sickle cell disease presents acutely with a rapidly progressive periorbital swelling. Haematomas frequently complicate the condition and, along with the inflammatory swelling, may lead to orbital compression syndrome. The condition is therefore sight-threatening, and necessitates prompt diagnosis and appropriate management for resolution without adverse sequelae. Imaging techniques are invaluable in the evaluation of patients. The majority of cases resolve with conservative treatment that includes steps to combat the vaso-occlusive crisis and use of systemic steroids under antibiotic cover.
KW - Management
KW - Orbital compression syndrome
KW - Orbital haemorrhage
KW - Orbital infarction
KW - Sickle cell disease
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U2 - 10.1038/eye.2001.248
DO - 10.1038/eye.2001.248
M3 - Article
C2 - 11827001
AN - SCOPUS:0035697096
SN - 0950-222X
VL - 15
SP - 774
EP - 780
JO - Eye
JF - Eye
IS - 6
ER -